Carcinoid heart disease remains a major cause of morbidity and mortality among patients with carcinoid syndrome and metastatic neuroendocrine tumors. Screening of all patients with N-terminal pro-B-type natriuretic peptide and transthoracic echocardiography is critical for early detection, as early symptoms and signs have low sensitivity for the disease. Cardiac surgery, in appropriate cases, is the only definitive therapy for advanced carcinoid heart disease, and it improves patient symptoms and survival. Management of carcinoid heart disease is complex, and multidisciplinary assessment of cardiac status, hormonal syndrome, and tumor burden is critical in guiding optimal timing of surgery.